Acquired lymphangiectasis after breast conservation treatment for breast cancer: Report of a case
From the Departments of Surgery1, Pathology2, Radiation Oncology3, and Medical Oncology4, School of Medicine, University of Uludag, 16059, Bursa, Turkey. firstname.lastname@example.org
Acquired lymphangiectasis is a dilatation of lymphatic vessels that can result as a complication of surgical intervention and radiation therapy for malignancy. Acquired lymphangiectasis shares clinical and histological features with the congenital lesion, lymphangioma circumscriptum. Diagnosis and treatment of these vesiculobullous lesions is important because they may be associated with pain, chronic drainage, and cellulitis. We describe patient who had these lesions after treatment for cancer. Although a number of treatment options are available, we have found cryosurgery and electrocautery to be particularly effective.
Acquired lymphangiectasis (AL) represent superficial lymphatic dilatation caused by a wide range of scarring processes and occurs as a consequence of lymphatic damage by an external cause, leading to obstruction of local lymphatic drainage. It has been reported after treatment of breast, vulva, cervix, skin, and lung cancers and it is sometimes termed acquired lymphangiomas . This condition has also been associated with metastatic lymph node invasion and obstruction, scleroderma, and scrofuloderma. Acquired lymphangiomas most commonly occur in adults as a late sequel of mastectomy and radiation therapy. Patients usually present with numerous translucent vesicles in a chronic lymphedematous area several years after surgery with or without radiation therapy . This report describes a case of acquired lymphangiectasis of the breast in 68-year-old woman. We discuss the clinical features, diagnosis, and treatment of acquired lymphangiectasis of the breast with a literature review.
A 68-year-old woman presented to our outpatient clinic for evaluation of multiple small vesicular lesions on the left breast. Three years earlier, she had had a breast conservative treatmentlumpectomy, chemotherapy, and radiation therapy for grade II invasive lobular carcinoma of the left breast. The breast lesions started to develop three years after breast conservative treatment. The patient had not had any significant lymphedema. On physical examination, the patient had numerous isolated and translucent vesicles and bullae of the inferior left nipple (Figs.1, 2). They measure 2 to 3 mm in diameter. No lymphedema was present also. We performed biopsy from lesions that showed numerous dilated lymphatics, edema and mononuclear inflammatory cells throughout the papillary and reticular dermis (Fig. 3). With these clinical and histological findings the patient was diagnosed as cutaneous lymphangiectasis.
Some of the lesions were treated with cryosurgery and others were drained and then treated with electrocautery. Cryotherapy freezes these lesions with liquid nitrogen (-195.8°:). Cryotherapy was applied to the skin twice for 20 seconds. Daily wound care consisted of gentle cleansing with povidone iodine and application of mupirocin ointment. At 8 weeks, the wound had completely re-epitheliazed. The wounds healed without scarring and there was no evidence of recurrence in subsequent 2 months.
Acquire lymphangiectasis is a condition in which dilated superficial lymphatics develop after damage to previously normal lymphatics. Acquired lymphangiectasis has been reported as clinically and histologically indistinguishable from congenital lymphangioma circumscriptum and is often reported in the literature as lymphangioma circumscriptum or acquired lymphangiomas [2, 3, 4, 5].
Cutaneous lymphangiectasis (CL) represents acquired vesicular dilatation of lymphatic channels secondary to an external cause with no evidence of true tumor formation. It has been associated mostly with the treatment of malignancies of breast and cervix and is often coexistent with lymphedema [1, 6, 7, 8]. There have also been reports of CL after treatment of myxoid chondrosarcoma and bronchial carcinoid [4, 9]. It has been documented after surgery alone, surgery and irradiation, and irradiation alone [8, 10]. Other reported cases without lymphedema include scarring from scrofuloderma, scleroderma, and cirrhosis [11, 12, 13].
Acquired lymphangiectasis has been reported in the literature with increased frequency in the past 20 years. This is most likely the result of increased surgical excision, surgery and radiation therapy for certain cancers. This condition has been documented to occur after surgical excision, surgery and irradiation and irradiation alone [3, 4, 10]. In our case, new lesions of lymphangiectasia ceased to develop after the conservative treatment. Although AL has been documented to occur after surgical intervention alone, we believe that x-ray therapy is a major contributor in its pathogenesis because the main site of injury of x-ray therapy is a major contributor in its pathogenesis, the site of injury from x-ray therapy is at the junction the fat and reticular dermis [8, 14]. This damage most likely causes fibrosis and lymphatic obstruction at the base of the reticular dermis and an accumulation of lymph fluid in the dermal lymphatics with resultant increased pressure . This can then lead to sacular dilation of the superficial lymphatic channels with subsequent vesicle formation. The cutaneous lesions can range from clear, fluid-filled blisters to smooth, flesh-colored nodules, often appearing along an incisional scar. Coexisting lymphedema is present in most patients with acquired lymphangioma. Patients can present with localized wetness or copious drainage of clear or milky fluid from ruptured vesicles. Clinically, lymphangiectasia consists of several clusters of translucent, thick-walled, fluid-filled vesicles. The vesicles typically measure 2-10 mm in diameter. The affected area appears to be speckled by numerous translucent vesicles with normal-appearing skin among the lesions. Some lesions may become pedunculated with a hyperkeratotic verrucous surface mimicking a wart. The physical findings of our case were the patient had numerous isolated and translucent vesicles of the inferior left nipple area. They measure 2-3 mm in diameter. On microscopic examination, large, dilated lymph vessels lined by a single layer of endothelial cells characterize lymphangiectasis. They are usually found in the papillary dermis and the reticular dermis. Involvement in the deeper dermis is rare. At times, acquired lymphangiectasis grow above the level of adjacent skin. The histological findings of our case were consistent with these features, the numerous dilated lymphatics, edema and mononuclear inflammatory cells throughout the papillary and reticular dermis.
Complications of AL include leakage of the vesicles, recurrent cellulites, and pain [15, 16]. Acquired lymphangiectasis is not precancerous, although there is one reported case of a squamous cell carcinoma arising in lymphangioma circumscriptum . This entity is different from the Stewart-Treves syndrome of lymphangiosarcoma developing in the setting of post-mastectomy lymphedema. Edematous and ecchymotic plaques progressing to red to purple nodules characterize lymphangiosarcoma .
Treatment for lymphangiectasis can be difficult, but it is important because of the risk that ruptured vesicles may provide a portal of entry for infection and subsequent cellulites. To prevent superinfection of ruptured vesicles, cleansing the affected area daily with topical antibacterial agents and applying mupirocin ointment or silver sulfadiazine cream are advisable. Many surgical treatment modalities have been advocated in the care of lymphangiectasis; these modalities include electrodesiccation, laser therapy, sclerotherapy, cryotherapy, and surgical excision. Daily compression through bandaging or hosiery in accessible areas has yielded acceptable results. In addition, laser therapy has also been shown to be an effective treatment. Various types of lasers have been used for this treatment, including the argon, tunable dye and CO&sub2; [1, 18, 19, 20]. There can be recurrences with all these treatments, and some may leave significant scarring. Our patient received treatment using the cryosurgery and some of them were drained and treated with electrocautery. Daily wound care consisted of gentle cleansing with povidone iodine and application of mupirocin ointment. At 8 weeks, the wound had completely re-epitheliazed. The wound was healed without any tissue scar and evidence of recurrence in following 2 months after re-epitheiazation.
Acquired lymphangiectasis is an important complication of radiation therapy and surgical intervention for malignancy and probably not as rare as once believed. Although this lesion has been described as histologically indistinguishable from lymphangioma circumscriptum, we believe these two conditions have true histologic differences in addition to their differing pathophysiology and clinical presentation. Acquired lymphangiectasis is an acquired dilation of lymphatics and can be associated with lymphedema after surgery and/or radiation therapy. Clinicians should be familiar with is presentation and pathogenesis since its complications can be significant. In addition, lesions are often cosmetically undesirable. The treatment is relatively simple, though recurrences are not uncommon.
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